Extrahepatic Sarcoidosis Causing Obstructive Jaundice and Recurrent Gastrointestinal Bleeding Due to Portal Hypertension

Abstract

Purpose: Extrahepatic sarcoidosis with granulomatous lymphnode involvement in porta hepatis is an extremely rare manifestation of sarcoidosis. So far no single case has been reported causing recurrent gastrointestinal bleeding secondary to extrahepatic sarcoidosis. This case report presents a 57-year-old woman who was diagnosed with sarcoidosis in 1998 when she was found to have uveitis and pulmonary nodules, subsequently treated with oral steroid. Eleven years later she presented to our clinic with nonspecific abdominal pain. CT abdomen showed pancreatic head mass which was thought to be malignant. She underwent exploratory laprotomy which showed soft tissue mass in porta hepatis encrouching portal vein. Biospy showed noncaseating granulomatous lymphnode confirming sarcoidosis. Patient declined steroid therapy. One year later, she developed pruritis and obstructive jaundice. Repeat CT abdomen showed growth of porta hepatic mass. She subsequently underwent ERCP for biliary decompression, but ultimately required biliary bypass surgery. One year later, abdominal CT scan revealed that the mass had progressed and was now causing extrinsic obstruction of both portal vein and hepatic artery. Patient had subsequnetly developed portal hypertension which lead to recurrent gastrointesital bleeding due to formation of jejunal varices at the site of biliary anstomosis. Initially, patient was treated conservatively with pulse dose steroid followed by maintenance high-dose steroid and mycophenolate mofetil which showed significant reduction in size of porta hepatic mass. However, she continued to have recurrent gastrointestinal bleeding and thus eventually required mesocaval shunt procedure to alleviate portal hypertension and to prevent recurrent gastrointestinal bleeding.